There are variable uses and some confusion about the distinction between plexiform neurofibroma and diffuse cutaneous neurofibroma, with some sources not clearly distinguishing between the two. Generally, plexiform neurofibromas are deeper lesions affecting nerves and plexus. The two may, however, co-exist 5.
Importantly it appears that diffuse cutaneous neurofibromas may not be as closely associated with neurofibromatosis type 1, nor have the same risk of malignant transformation 1-6. The distinction is thus important.
Diffuse neurofibromas mainly affect children and young adults 1.
These tumours most frequently present with overgrowth of the subcutaneous tissues typically of the head and neck, trunk and extremities1.
Diffuse cutaneous neurofibromatosis cause plaque-like elevation of the skin with thickening of the entire subcutis but without nodular masses 1,3,4.
Diffuse cutaneous neurofibromas usually have fairly non-specific signal intensity 1:
- T1: iso- to hypointense to skeletal muscle
- T1 C+: usually vivid contrast enhancement
- T2: hyperintense to skeletal muscle
Treatment and prognosis
Again, unlike plexiform neurofibromas, malignant transformation is uncommon 1,4.
- 1. Hassell DS, Bancroft LW, Kransdorf MJ, Peterson JJ, Berquist TH, Murphey MD, Fanburg-Smith JC. Imaging appearance of diffuse neurofibroma. AJR. American journal of roentgenology. 190 (3): 582-8. doi:10.2214/AJR.07.2589 - Pubmed
- 2. Murphey MD, Smith WS, Smith SE et-al. From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19 (5): 1253-80. Radiographics (citation) - Pubmed citation
- 3. Dähnert WF. Radiology Review Manual. (2007) ISBN:0781766206. Read it at Google Books - Find it at Amazon
- 4. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised" ISBN: 9789283244929
- 5. Marta C. Cohen, Irene Scheimberg. Essentials of Surgical Pediatric Pathology. ISBN: 9781316124154
- 6. Mark J. Kransdorf, Mark D. Murphey. Imaging of Soft Tissue Tumors. ISBN: 9780781747714